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Multiple myeloma and plasmacytoma: cancer of the plasma cells
Multiple myeloma, also known as Kahler's disease, is a malignant tumor that arises from the degeneration of a single plasma cell. Their clones spread in the bone marrow, which is why the disease is also known as bone marrow cancer. The individual forms are very different, with the appearance of only a single tumor being the special form of the plasmacytoma. Advancing medical knowledge has led to a multitude of new treatment options and an improved prognosis. However, the disease is considered incurable.
A brief overview
The following overview summarizes the most important facts about the complex clinical picture of multiple myeloma or plasmacytoma. The following article also contains detailed and current information about the disease.
- definition: Multiple myeloma or plasmacytoma is a malignant cancer from the group of low-grade non-Hodgkin lymphomas. Starting from a single degenerated plasma cell, there is an uncontrolled proliferation of these cells that infiltrate the bone marrow. Many tumor sites are typical and spread to different locations. Seldom does a single tumor grow, then one speaks of a plasmacytoma.
- Symptoms: Complaints do not always occur (at the onset of illness). Frequently, unspecific general symptoms such as bone pain, fever, weight loss or night sweats initially appear. Typical symptoms of anemia, increased susceptibility to infection, foaming urine (Bence-Jones proteinuria) and unusual bleeding often occur as a result of the abnormal plasma cells. Other serious consequences and complications are possible.
- causes: The causes of the disease are still unknown. A certain hereditary component is suspected, but the body's own processes and possibly harmful environmental influences also play a role in the development of cancer.
- diagnosis: It is not uncommon for a lack of or unspecific symptoms to lead to random findings in blood and urine tests. However, special diagnostic procedures are necessary in order to be able to make a precise diagnosis. This includes precise detection of the light chain proteins in serum and urine, a bone marrow biopsy and imaging diagnostics.
- treatment: The disease is not curable, but medical advances have greatly improved the prognosis and treatment options. Today's treatment concepts basically involve chemotherapy including so-called “new substances”, high-dose chemotherapy with stem cell transplantation and radiation.
- Naturopathic treatment: Alternative healing methods offer many options for supportive cancer therapy, in which the psyche and the immune system are strengthened, metabolic processes are regulated and side effects are reduced. Mistletoe therapy is used in particular to improve the tolerance of chemotherapy.
- Current state of research: The clinical picture of multiple myeloma is the focus of many research activities. Developments in recent years in particular have given those affected hope for better treatment options and prognoses through new drugs.
Multiple myeloma or plasmacytoma is a malignant cancer from the group of low-grade non-Hodgkin's lymphomas (malignant diseases of the lymphatic system). This affects the B lymphocytes, which belong to the cell group of leukocytes (white blood cells), or the plasma cells that result from further cell differentiation. Low-grade (indolent) lymphomas are chronic in nature and grow relatively slowly. As a rule, the disease is not curable, but it can be treated.
Starting from a single degenerated plasma cell (myeloma cell) in the bone marrow, there are uncontrolled proliferation of identical plasma cells (clones). These constantly produce monoclonal, inoperable immune bodies (immunoglobulins), which are also known as "paraproteins". This leads to a weakened immune system and healthy blood-forming cells can be displaced in the bone marrow. Other possible consequences include anemia, decreased platelets and increased bone loss (osteolysis).
The disease is rather rare, but is one of the most common bone and bone marrow tumors in western industrialized countries. Most diagnoses are made in older age (70th year of life) and men are at higher risk of illness (3: 2). Various factors are attributed to the increasing diagnosis rate in the past decades.
The first description of the disease goes back to the English doctors William Macintyre (1792-1857) and Henry Bence Jones (1813-1873), who examined the first known patient case by Thomas Alexander McBean. Bence Jones was able to detect unusual proteins in the patient's urine for the first time, which are now known as Bence Jones proteins. If these so-called light chain proteins (Bence-Jones proteinuria) are found these days, this primarily indicates a malignant increase in B lymphocytes and thus often a multiple myeloma.
In 1873, the Russian surgeon Dr. J. von Rustizky entered the term multiple myeloma, thereby describing the presence of multiple plasma cell lesions in the bone. Symptoms similar to those of other doctors have also been described, the names of which partly stand for synonymous names of the same disease. In 1889, for example, the Austrian internist Otto Kahler published a detailed clinical description of multiple myeloma under the name "Kahler disease". Huppert, Bozzolo, Ramón y Cajal, Wright and Weber also made a name for themselves through early investigations into the symptoms of this clinical picture.
Synonyms and terminology
The terms multiple myeloma and plasmacytoma are often used interchangeably, but refer to two different clinical pictures. Starting from the degeneration of a single plasma cell, its clones multiply and spread in the bone marrow. Many tumor sites are typical and spread diffusely (multiple myeloma). However, if there is only a single focus, it is the special and rare form of the so-called (solitary) plasmacytoma. Other less common synonyms include Kahler disease (Kahler disease) after Otto Kahler and Huppert disease after Karl Hugo Huppert.
Differentiation from the clinical picture of multiple myeloma are diseases that count as preliminary stages, such as so-called asymptomatic (smoldering) multiple myeloma and monoclonal gammopathy of unclear significance, which represents a benign condition. Relatively large overlaps also exist with lymphoplasmocytic lymphoma, which underlies Waldenström's macroglobulinemia (Waldenström's disease).
The first complaints are often rather unspecific and diverse. It is not uncommon for those affected to be symptom-free initially and when diagnosing. General symptoms, which can also appear in the early stages of the disease, mainly include bone pain (back, ribs, hips, extremities) and the so-called B symptoms fever, weight loss and night sweats.
The bone pain mostly arises from the infestation and the increasing spread of the tumor cells, caused by nerve irritation and the pressure on surrounding nerves. (Small) spontaneous fractures, which can be caused by a breakdown of the bone (osteolysis), also cause pain.
Due to the infiltration of the bone marrow and the excessive production of the monoclonal plasma cells and paraproteins, further complaints can occur in the course of the disease. The typical signs of multiple myeloma are as follows:
- Symptoms of anemia such as chronic fatigue, shortness of breath when under stress, headache, irritability and pallor.
- An increased susceptibility to infections (especially urinary tract infections), partly due to the lack of healthy white blood cells (leukopenia) and functional antibodies.
- A foaming urine, as a sign of Bence-Jones proteinuria and the increased excretion of light chain proteins.
- Unusual bleeding (nosebleeds, heavy menstrual bleeding) and a tendency to bruise (hematoma) due to disorders in blood formation (platelet deficiency).
In the advanced stage of the disease, the function of the kidneys in particular can be impaired by the amount of proteins produced and the excess calcium released during osteolysis (hypercalcaemia).
Hyperviscosity syndrome is one of the more rare complications, whereby the fluidity of the blood is reduced by the high protein content in such a way that dizziness, angina pectoris, thrombosis or other serious consequences can occur. If the light chain proteins combine with other proteins, amyloid proteins can be formed, which in rare cases can cause myeloma-associated AL amyloidosis (light chain amyloidosis). Deposits of the proteins in various tissues and organs (especially kidneys, heart, gastrointestinal tract, liver and nerves) can severely impair their functions.
The causes are still largely unknown, not least because multiple myeloma has very heterogeneous genetic and clinical characteristics. A variety of different chromosomal abnormalities that have been discovered in connection with the disease appear to have a significant impact on the course of the disease. A certain hereditary component is suspected, but it is not an inherited disease.
The body's own processes such as the growth and differentiation of cells, cell interactions and the immune system play a crucial role in the development and spread of multiple myeloma.
In addition, it is being discussed whether various harmful and possibly carcinogenic environmental influences, such as radiation or pesticides, promote the development of multiple myeloma.
It often takes several months between the first symptoms of the disease and the diagnosis. In about 20 percent of those affected, random findings from blood and urine tests also lead to the diagnosis of the disease. For example, a very high blood sedimentation rate (“fall”) or a high protein content in the blood or urine provide first indications of the presence of multiple myeloma. If you suffer from bone pain, this can also be a possible sign. In any case, in addition to a detailed medical history and physical examination, special diagnostic procedures must be carried out.
Laboratory tests of blood and urine samples
After a large blood count, protein electrophoresis of the serum and urine is the first screening method for the detection of a monoclonal protein. Further tests serve to determine the exact type of protein (immunofixation electrophoresis) and to measure the free light chains in the serum. Urine (24-hour urine collection) should always be examined as well, since the light chain proteins are excreted when the kidney function is intact and are therefore not detectable in the serum. When diagnosing, it is important to make a precise distinction between the preliminary stages and forms of multiple myeloma.
Bone marrow examination
A bone marrow biopsy is an essential part of further diagnostics. As a rule, a sample of the bone marrow is obtained from the posterior iliac crest, which is subsequently used for histological examinations. The changed cancer cells can be recognized, as well as infiltration patterns (diffuse or nodular) and the degree of infiltration.
Imaging procedures are used in the initial diagnosis and in the course of the disease. First, conventional X-rays are taken, whereby the pathological involvement of multiple myeloma is primarily visible on the skull and spine. The x-rays then show typical honeycomb and egg-shaped osteolyses. A finding of multiple osteolysis in the skull is also called a shotgun skull. If the picture shows an osteoporosis, it is a diffuse infiltration. The latter cannot be distinguished radiologically from age-related osteoporosis.
Computed tomography is also used to determine, in particular, poor bone dissolution. Magnetic resonance imaging is mainly used to assess tumors in the area of the spine and outside the bone (extraosseous).
One speaks of a (symptomatic) multiple myeloma in the actual sense only if more than 10 percent monoclonal plasma cells were found in the bone marrow and according to the so-called CRAB criteria of the International Myeloma Working Group (IMWG) there is at least secondary organ damage or a corresponding proof of the extended SLiM criteria. The CRAB criteria include hypercalcemia, renal failure, anemia and bone dissolution, while the SLiM criteria relate to the ratio of light chain proteins and the amount of plasma cells in the bone marrow and their visible accumulation in magnetic resonance imaging.
The most important criteria for a plasmacytoma are the detection of less than ten percent monoclonal plasma cells in the bone marrow and a single radiologically detectable focus of disease, with a simultaneous lack of altered protein in the blood serum and in the urine.
In the past, the need for therapy according to the staging of Durie and Salmon was determined. According to recent medical findings, the need for (immediate) therapy after diagnosis is currently being decided using the criteria of the IMWG.
The disease is considered incurable, but in recent years, major medical advances have greatly improved the prognosis and treatment options. The aim of every treatment is to stabilize the patient, alleviate the symptoms and extend life through the longest possible remission (suppression of the disease).
Nowadays, different methods and substances are available for the treatment. Depending on the patient's case, a decision is made individually as to which therapeutic measures make sense. The age, the general state of health, the form of corresponding complaints and various risk factors are also taken into account when making decisions.
Possible treatment concepts are fundamentally chemotherapy including so-called "new substances", high-dose chemotherapy with stem cell transplantation and radiation.
The chemotherapies are designed to kill myeloma cells and thus suppress the disease throughout the body (systemically). Certain cytostatics, so-called alkylating agents (e.g. melphalan), have proven to be particularly effective. A very high dosage of the chemotherapeutic agents could achieve complete remission in a relatively large number of cases. However, side effects can be expected with every chemotherapy and a high-dose therapy usually leads to a severe impairment of the bone marrow. In order to counteract this damage, high-dose chemotherapy is often combined with the administration of growth factors or a stem cell transplant. The body's own cells (autologous) or stem cells from a donor (allogeneic) can be used.
In principle, radiation can only be carried out on certain parts of the body (local). With the help of radiation, degenerate cells are said to lose their ability to divide in order to stop tumor growth. Radiation therapy is primarily used to treat bone pain.
Under certain conditions, newer substances are used either in combination with other therapies or as an alternative treatment method. These drugs include so-called immunomodulating substances (thalidomide, pomalidomide, lenalidomide), proteasome and histone deacetylase inhibitors (bortezomib and panobinostat) and the antibodies elotuzumab and daratumumab. Interferon and bisphosphonates are also used. Not all novel medicines have been adequately tested for their effectiveness and are therefore not yet officially approved.
The treatment of accompanying symptoms can also be important in therapy and should be clarified individually with the treating specialist.
Strengthening the body's own self-healing powers can have a positive influence on the course of the disease and mitigate the side effects of chemotherapy. Numerous methods from the field of naturopathy can be used.
In accompanying biological cancer therapy, various methods and natural remedies are combined in order to strengthen the psyche and body, regulate the metabolism and activate the immune system. With mistletoe therapy in particular, an improvement in the immune system and general quality of life as well as the special reduction in fatigue, nausea and depressive moods (side effects of chemotherapy) are proven. Additional naturopathic treatment to fight infections, for example by means of microbiological therapies, can also be considered.
The list of all possible naturopathic treatments for supporting cancer treatment, also in the case of multiple myeloma or plasmacytoma, is long and should be discussed in individual cases with expert experts.
A surprising therapeutic success has so far been assigned to the ginger turmeric (turmeric). In Far Eastern medicine, the active ingredient curcumin from the plant turmeric (Curcuma longa) has long been considered an antioxidant, antiseptic and anti-inflammatory. In general, turmeric is healthy and often healing. Many scientific studies have confirmed this. According to a case report published in the British Medical Journal in April 2017, the active ingredient curcumin is also said to have great potential for the remission of multiple myeloma.
Current state of research
The clinical picture of multiple myeloma is the subject of much international research. There are also several study groups and competence networks in Germany that advance research on new forms of therapy and diagnostic procedures and enable basic research. For example, the prognosis of those affected has been significantly improved in recent years, particularly through new drugs and treatment options.
Researchers recently made a breakthrough that leaves those affected with a hopeful message: Drugs kill cancer cells without side effects. As the Pharmaceutical newspaper reported, a gradual (full) approval of new drugs is expected in the next few years. (jvs, cs; updated on January 14th, 2019)
Author and source information
This text corresponds to the requirements of the medical literature, medical guidelines and current studies and has been checked by medical doctors.
Dr. rer. nat. Corinna Schultheis
- ONKO Internet portal of the German Cancer Society: Multiple myeloma: basic information for patients and relatives (access: 03.07.2019), krebsgesellschaft.de
- International Myeloma Foundation (IMF, International Myeloma Foundation): Patient Handbook Edition 2017 (accessed: 03.07.2019), myeloma.org
- Myeloma Germany e. V .: Description of multiple myeloma (accessed: 03.07.2019), myelom-deutschland.de
- German Cancer Aid Foundation: Guide to plasmacytoma / multiple myeloma (as of April 2018), krebshilfe.de
- Gerecke, Christian / Fuhrmann, Stephan / Strifler, Susanne / u.a .: Diagnostics and therapy of multiple myeloma. Dtsch Arztebl Int 2016, aerzteblatt.de
- Amboss GmbH: Multiple myeloma (Kahler disease) (access: 03.07.2019), amboss.com
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- Mayo Clinic: Multiple myeloma (accessed: 03.07.2019), mayoclinic.org
- National Health Service UK: Overview - Multiple myeloma (accessed: 03.07.2019), nhs.uk
- German Society for Hematology and Medical Oncology: Guideline for Multiple Myeloma, as of May 2018, onkopedia.com
ICD codes for this disease: C90ICD codes are internationally valid encodings for medical diagnoses. You can find e.g. in doctor's letters or on disability certificates.